1
ABSTRACTS MITRAL VALVE PROLAPSE SYNDROME: ANTHROPOMETRIC, SEXUAL AND CLINICAL FEATURES Bernard Kinp MD; Harisios Boudoulas, MD, FACC; Mary E. Fontana, ?Q', FACC; Charles F. Wooley, MD, FACC; The Ohio State University College of Medicine, Columbus, Ohio The spectrum of the mitral valve prolapse (MW) Syn- drome is incgmpletely defined. Three hundred thirteen patients (86 M, mean age 34.42 14 (SD) yrs and 227 F, mean age 34.3+ 15 yrs) with auscultatory MVP were seen for sympt&s (SX) or murmur evaluation from 1963-1977. Height/weight ratio was greater in MVP compared to nor- mals (M .46f .04 (SE) vs .42? .03, ~0.01, and F .54_ .02 "S .47f .04, w.01). Arm span/height ratio was 1.022 .03 in M and 1.012 ,03 in F. ?+a~ age of Sx onset was the same in M and F (30.6 vs 29.9 yrs). Chest pain was the initial complaint in 50% M vs 36% F (p&01). Conversely, 42% F presented initi- ally with palpitations compared to 21% M (F&01). Neuro- logic 'Sx (including syncope), an initial manifestation in 9.5%, were present sometime in 15% of all. Chest pain was .noted by 60% of both sexes. Palpitation (75%), fa- tigue (48%), dyspnea (34%) and arrhythmias (54%) were more fi-equent in F than M (48%, 28%, 16%, 34%, respec- tively, w.01). The number of Sx was greater in F than M (2.92 .l "s 1.9 _+ .l, p(.Ol); 10.5% of F and 16% of M were asymptomatic. Distinctive anthropometric features; sexual diff- erences in habitus, number and type of symptoms; pre- viously documented (by this group) evidence of increased adrenetgic activity; reported sleep disorders and auto- nomic dy3function - permit definition of the systemic process, the MW syndrome, and support the concept that WP syndrome is a constitutional-neuroendocrine-cardio- vasctilar process. MITRAL VALVE PROLAPSE : POSSIBLE MANIFESTATION OF A CONGE- NITAL NEURO-ECTO-MBSODERMAL HISTODYSPLASIA- Philippe Jean-Louis -Chef de clinique -Service cardiologie Maurice Pent)-Hopital Croix-Rousse -Lyon 69717 - France. ;k&yosology of mitral valve prolapse (MVP) is as yet unk- To evaluate the possibility that MVP may be a mani- testation of a congenital histodysplasia,We studied 110 men(M) women(W)w MVP proved by phone(P) and echocar- diography(E) without any other echocardiographic abnorma- lity -I)Psychologicaltest-Rickels'rating scale(RRS)-was randomly administered to 60 MVP(MW) and 3O(MW) with acqui- red valvular heart disease;MVP scored significantly hi- gher on the depression, somatization, sleep disorder, an- xiety scales of the RRS.The global psychopathological sco- re was significantly higher in MVP(P<O,Ol).II)23% of the women with psychosomatic digestive disorders had MVP pro- ved by P and E(prospective study). III) Dermatological examination was performed in 50 MVP(MW) : 28 had ichthyo- sis (xerodermia) and/or cafP au lait spots(24) and/or po- lythelia(2)and/or lentigines(?l)and/or lipomatosis(3)(pro- spective research of MVP in patients with these epidermal manifestations is being performed)-1V)Vertebrothoracic ab- normalities were comparable to those previously described and found in lOO%(scoliosis and/or straight back and/or pectus excavatum etc.).Perhaps the link between MVP, der- matological; psychopathological and vertebrothoracic ab- normalities could be explained by a single neural crest and mesodermal embryonic tissue defect during the 4th to 8th week of embryonic development.(MVP has an autosomal dominant mode of inheritance and is known to he associated with connective: tissue disorders such as Marfan's syndr- om, Ehlets Danlos syndrom etc.).This study suggests that MVP is a manifestation of congenital neuro-ecto-mesoder- ma1 histodys plasia-The neurogenic factor (central and pe- ri heral ) would explain many symtoms and features associa te$ with MVP. TUESDAY, MARCH 71, 1980 PM SUDDEN DEATH: TREATMENT OF VENTRICULAR TACtiYARRHYTHiWA 2:00-5:30 RADIONUCLIDE VENTRICULOGRAPHY IN 121 SURVIVORS OF OUT-OF- HOSPITAL VENTRICULAR FIBRILLATION James L. Ritchie, MD, FACC; Gene B. Trobaugh, MD; Alfred P. Hallstrom, PhD; Glen W. Hamilton, MD; Leonard A. Cobb, MD, FACC; Seattle Veterans Administration Hospital and Harborview Hospital. University of Washington, Seattle. Wa. Radionuclide left ventriculography was performed at rest (R) in 121. and at R and maximal supine bicycle exercise (EX) in 66 survivors of out-of-hospital ventricular fib- rillation (VF) attributed to coronary artery disease (CAD) (103 male, 18 female, mean age 58). Mean R ejection fraction (LVEF) overall was .41+.17 (n=121). Resting LVEF was below .30 in 36% of patients, .31-.50 in 312, and >.50 in 33%. Regional dyskinesis was present in 14%. akinesis in 37%, and hypokinesis in 29%. Mean EX-LVEF was .39?.17 (compared to mean R-LVEF of .43+.17; n=66). At an average of 13.2 months of known followup, 10 of 118 (8%) patients had cardiac death or repeat VF. Mean R- LVEF in non-survivors was .30?.16 vs. .42+.17 (pc.015) in survivors. Non-survivors had more regions of akinesis or dyskinesis (7/8. 88%) than survivors (39/82. 48%; pc.001). The magnitude of LVEF depression with exercise was not greater in non-survivors (.03 VS. .04, p=NS). We conclude that: 1) Marked depression of LV function is not the common denominator of out-of-hospital VF; two thirds of patients are either normal (EF>.50) or have moderate (EFs.30) LV dysfunction. Many such patients are thus possible candidates for further medical or surgical therapy. 2) Recurrent VF/death is associated with a low R-LVEF and more severe wall motion abnormalities, but not with the extent of EX-induced LV dysfunction. LACK OF EFFECT OF ASSOCIATED MYOCARDIAL INFARC- TION ON LONG-TERM SURVIVAL OF OUT-OF-HOSPITAL VEN- TRICULAR FIBRILLATION. Bim Sharma, MDi Morrison Hodges, MD, FACC; Richard W. Asinger, MD; Gary Francis, MD, FACC. Hennepin County Me+ ical Center & the University of Mimesota, Minneapolis, Minne- sota. Currently the prognosis of patients who survive out-of-hospital ventricular fibrillation (OHVF) is predicated on whether or not there is an attendant myocerdial infarction. This report concerns the long term survival (1974-1978) of 90 patients (78 males and 12 females) who were discharged fern the hospital ambulatory with full mental faculties after OHVF. Average age was 58.3 Years (range 15-83). The occurence of acute myocardial infarcticn at the time of OHVF was determined by serial ECG, serial cardiac specific enzyme (CK-MB) and Tc pyrophosphate scans. Three groups emerged: 1) patients who sustained definite acute myo- cardial infarctim (45 patients); 2) patients with no acute myo- cardial infarction (32 patients) end 3) an uncertain group (13 patients), Follow-up of the 90 patients from the period 1974-1976 revealed that 16 of 45 patients in acute myocardial infarction grog (35.6%), 9 of 32 in no acute myocardial infarctim group (28.1%) and 4 of 13 uncertain patients (30.8%) sustained sudden death. The survival curve of all 90 patients indicated the survival rate at 1, 2, 3, 4 yrs to be 78%, 64%, 53% and 43% respectively. The survival curves of acute myocardial infarctim, no acute myocardial infarction, and uncertain groups were not different ftnrn each other. This study differs from others in the following wap: a) There was no uncertain group in previous studies. In this study 13 pts (uncertain group) had no clear-cut evidence which allowed either diagnosis or exclusion of acute myocardial infarction; and b) The acute myocardial infarction group had as a high mortality as those without. Thus, in our study, the occurrence of acute myocardial infarctim et the time of OHVF does not seem to affect long-term survival. February 1980 The American Journal of CARDIOLOGY Volume 45 443

Mitral valve prolapse syndrome: Anthropometric, sexual and clinical features

Embed Size (px)

Citation preview

ABSTRACTS

MITRAL VALVE PROLAPSE SYNDROME: ANTHROPOMETRIC, SEXUAL

AND CLINICAL FEATURES Bernard Kinp MD; Harisios Boudoulas, MD, FACC; Mary E.

Fontana, ?Q', FACC; Charles F. Wooley, MD, FACC; The Ohio State University College of Medicine, Columbus, Ohio

The spectrum of the mitral valve prolapse (MW) Syn- drome is incgmpletely defined. Three hundred thirteen

patients (86 M, mean age 34.42 14 (SD) yrs and 227 F, mean age 34.3+ 15 yrs) with auscultatory MVP were seen for sympt&s (SX) or murmur evaluation from 1963-1977. Height/weight ratio was greater in MVP compared to nor- mals (M .46f .04 (SE) vs .42? .03, ~0.01, and F .54_ .02

"S .47f .04, w.01). Arm span/height ratio was 1.022 .03

in M and 1.012 ,03 in F. ?+a~ age of Sx onset was the same in M and F (30.6 vs

29.9 yrs). Chest pain was the initial complaint in 50%

M vs 36% F (p&01). Conversely, 42% F presented initi- ally with palpitations compared to 21% M (F&01). Neuro- logic 'Sx (including syncope), an initial manifestation in 9.5%, were present sometime in 15% of all. Chest pain

was .noted by 60% of both sexes. Palpitation (75%), fa-

tigue (48%), dyspnea (34%) and arrhythmias (54%) were more fi-equent in F than M (48%, 28%, 16%, 34%, respec-

tively, w.01). The number of Sx was greater in F than M (2.92 .l "s 1.9 _+ .l, p(.Ol); 10.5% of F and 16% of M were asymptomatic.

Distinctive anthropometric features; sexual diff- erences in habitus, number and type of symptoms; pre- viously documented (by this group) evidence of increased adrenetgic activity; reported sleep disorders and auto- nomic dy3function - permit definition of the systemic process, the MW syndrome, and support the concept that WP syndrome is a constitutional-neuroendocrine-cardio- vasctilar process.

MITRAL VALVE PROLAPSE : POSSIBLE MANIFESTATION OF A CONGE- NITAL NEURO-ECTO-MBSODERMAL HISTODYSPLASIA-

Philippe Jean-Louis -Chef de clinique -Service cardiologie Maurice Pent)-Hopital Croix-Rousse -Lyon 69717 - France.

;k&yosology of mitral valve prolapse (MVP) is as yet unk-

To evaluate the possibility that MVP may be a mani- testation of a congenital histodysplasia,We studied 110 men(M) women(W)w MVP proved by phone(P) and echocar- diography(E) without any other echocardiographic abnorma- lity -I)Psychologicaltest-Rickels'rating scale(RRS)-was randomly administered to 60 MVP(MW) and 3O(MW) with acqui- red valvular heart disease;MVP scored significantly hi- gher on the depression, somatization, sleep disorder, an- xiety scales of the RRS.The global psychopathological sco- re was significantly higher in MVP(P<O,Ol).II)23% of the women with psychosomatic digestive disorders had MVP pro- ved by P and E(prospective study). III) Dermatological examination was performed in 50 MVP(MW) : 28 had ichthyo- sis (xerodermia) and/or cafP au lait spots(24) and/or po- lythelia(2)and/or lentigines(?l)and/or lipomatosis(3)(pro- spective research of MVP in patients with these epidermal manifestations is being performed)-1V)Vertebrothoracic ab- normalities were comparable to those previously described and found in lOO%(scoliosis and/or straight back and/or

pectus excavatum etc.).Perhaps the link between MVP, der- matological; psychopathological and vertebrothoracic ab- normalities could be explained by a single neural crest and mesodermal embryonic tissue defect during the 4th to 8th week of embryonic development.(MVP has an autosomal dominant mode of inheritance and is known to he associated with connective: tissue disorders such as Marfan's syndr- om, Ehlets Danlos syndrom etc.).This study suggests that MVP is a manifestation of congenital neuro-ecto-mesoder- ma1 histodys plasia-The neurogenic factor (central and pe- ri heral ) would explain many symtoms and features associa te$ with MVP.

TUESDAY, MARCH 71, 1980 PM SUDDEN DEATH: TREATMENT OF VENTRICULAR TACtiYARRHYTHiWA 2:00-5:30

RADIONUCLIDE VENTRICULOGRAPHY IN 121 SURVIVORS OF OUT-OF- HOSPITAL VENTRICULAR FIBRILLATION

James L. Ritchie, MD, FACC; Gene B. Trobaugh, MD; Alfred P. Hallstrom, PhD; Glen W. Hamilton, MD; Leonard A. Cobb, MD, FACC; Seattle Veterans Administration Hospital and Harborview Hospital. University of Washington, Seattle. Wa.

Radionuclide left ventriculography was performed at rest (R) in 121. and at R and maximal supine bicycle exercise (EX) in 66 survivors of out-of-hospital ventricular fib- rillation (VF) attributed to coronary artery disease (CAD) (103 male, 18 female, mean age 58). Mean R ejection fraction (LVEF) overall was .41+.17 (n=121). Resting

LVEF was below .30 in 36% of patients, .31-.50 in 312, and >.50 in 33%. Regional dyskinesis was present in 14%. akinesis in 37%, and hypokinesis in 29%. Mean EX-LVEF was .39?.17 (compared to mean R-LVEF of .43+.17; n=66). At an average of 13.2 months of known followup, 10 of 118

(8%) patients had cardiac death or repeat VF. Mean R- LVEF in non-survivors was .30?.16 vs. .42+.17 (pc.015) in survivors. Non-survivors had more regions of akinesis or dyskinesis (7/8. 88%) than survivors (39/82. 48%; pc.001). The magnitude of LVEF depression with exercise was not greater in non-survivors (.03 VS. .04, p=NS).

We conclude that: 1) Marked depression of LV function is not the common denominator of out-of-hospital VF; two thirds of patients are either normal (EF>.50) or have moderate (EFs.30) LV dysfunction. Many such patients are thus possible candidates for further medical or surgical therapy. 2) Recurrent VF/death is associated with a low R-LVEF and more severe wall motion abnormalities, but not with the extent of EX-induced LV dysfunction.

LACK OF EFFECT OF ASSOCIATED MYOCARDIAL INFARC- TION ON LONG-TERM SURVIVAL OF OUT-OF-HOSPITAL VEN- TRICULAR FIBRILLATION. Bim Sharma, MDi Morrison Hodges, MD, FACC; Richard W. Asinger, MD; Gary Francis, MD, FACC. Hennepin County Me+ ical Center & the University of Mimesota, Minneapolis, Minne- sota.

Currently the prognosis of patients who survive out-of-hospital ventricular fibrillation (OHVF) is predicated on whether or not there is an attendant myocerdial infarction. This report concerns the long term survival (1974-1978) of 90 patients (78 males and 12 females) who were discharged fern the hospital ambulatory with full mental faculties after OHVF. Average age was 58.3 Years (range 15-83). The occurence of acute myocardial infarcticn at the time of OHVF was determined by serial ECG, serial cardiac specific enzyme (CK-MB) and Tc pyrophosphate scans. Three groups emerged: 1) patients who sustained definite acute myo- cardial infarctim (45 patients); 2) patients with no acute myo- cardial infarction (32 patients) end 3) an uncertain group (13 patients), Follow-up of the 90 patients from the period 1974-1976 revealed that 16 of 45 patients in acute myocardial infarction grog (35.6%), 9 of 32 in no acute myocardial infarctim group (28.1%) and 4 of 13 uncertain patients (30.8%) sustained sudden death. The survival curve of all 90 patients indicated the survival rate at 1, 2, 3, 4 yrs to be 78%, 64%, 53% and 43% respectively. The survival curves of acute myocardial infarctim, no acute myocardial infarction, and uncertain groups were not different ftnrn each other.

This study differs from others in the following wap: a) There was no uncertain group in previous studies. In this study 13 pts (uncertain group) had no clear-cut evidence which allowed either diagnosis or exclusion of acute myocardial infarction; and b) The acute myocardial infarction group had as a high mortality as those without. Thus, in our study, the occurrence of acute myocardial infarctim et the time of OHVF does not seem to affect long-term survival.

February 1980 The American Journal of CARDIOLOGY Volume 45 443